What is PAH in Systemic Sclerosis (SSc)?

The connection between PAH and Systemic Sclerosis

Pulmonary arterial hypertension (PAH) occurs in approximately one in seven scleroderma patients.¹ Pulmonary complications, namely PAH and pulmonary fibrosis, are a common cause of death in SSc patients.² Unfortunately, with such symptoms as breathlessness, fatigue on exercise and syncope, it can masquerade as other respiratory or cardiac complaints with devastating consequences.^{3, 4} Furthermore, some patients may have already reduced daily activities (i.e. mobility problems) and breathlessness may not be the first symptom. Therefore, PAH needs to be considered in the daily management of SSc patients and screening is the key to establishing early diagnosis. International guidelines recommend screening by Doppler echocardiography annually and/or in the presence of unexplained breathlessness.^{1, 4, 5} Effective treatments are now available which may improve quality of life and exercise capacity, slow disease progression and improve long-term outcome.⁶
 
If you suspect PAH, consider referring the patient to a centre with expertise in PAH.

References
1. Hachulla E, Coghlan JG. A new era in the management of pulmonary arterial hypertension related to scleroderma: endothelin receptor antagonism.  Ann Rheum Dis 2004; 63:1009-14.
2. Steen V et al. Changes in causes of death in systemic sclerosis, 1972-2002. Ann Rheum Dis 2007; 940-944
3. Runo JR, Loyd JE. Primary pulmonary hypertension.  Lancet 2003; 361:1533-44.
4. McGoon M, Gutterman D, Steen V et al.  Screening, early detection, and diagnosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 2004; 126:14S−34S.
5.Galie N, Torbicki A, Barst R et al. Guidelines on diagnosis and treatment of pulmonary arterial hypertension. The Task Force on Diagnosis and Treatment of Pulmonary Arterial Hypertension of the European Society of Cardiology. Eur Heart J. 2004; 25:2243-78. 
6.Badesch DB, Abman SH, Ahearn GS et al. Medical therapy for pulmonary arterial hypertension.  ACCP evidence-based clinical practice guidelines.  Chest 2004; 126:35S-62S.